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'''Pervasive refusal syndrome''' ('''PRS''') is a rare but serious child [[psychiatric disorder]] that was first described by Bryan Lask and colleagues in 1991.<ref name=adc66_866/> As of 2011, it is not included in the standard [[Classification of mental disorders|psychiatric classification systems]].<ref name=ep26_1_291/>
'''Pervasive refusal syndrome''' ('''PRS''') is a rare but serious child [[psychiatric disorder]] that was first described by Bryan Lask and colleagues in 1991.<ref name=adc66_866/> As of 2011, it is not included in the standard [[Classification of mental disorders|psychiatric classification systems]].<ref name=ep26_1_291/>. PRS is the name allotted to a disorder in which children
have abandoned their involvement in all phases of their life. It's characterized by refusal

to eat, drink, talk, walk or self-care, and a firm resistance to treatment <ref name=Parents>{{cite journal|title=Pervasive Refusal Syndrome: A Parent's Perspective|journal=Sage Journals|year=2001|volume=6|issue=455|url=http://ccp.sagepub.com/content/6/3/455}}</ref>. PRS is very rare and its cause is unclear, but its severity makes it [[Life-threatening disease|life-threatening]]. The disorder usually begins with a '[[virus]]', or the child having a 'pain', that results in the need for consulting a doctor or going to the hospital, even though no substantial cause can be found. PRS starts slowly, but the child then worsens quickly becoming reluctant or not capable to do anything for themselves <ref name=Parents /> . They originally refuse to accept others caring for them, or helping them eat, and are very [[Depression (mood)|depressed]] and distraught. It is not guaranteed that recovery will take place, and it is a lengthy and complex process, involving specialist medical care <ref name=Parents /> . Nevertheless, once the patient is healthy, relapse is very infrequent.
PRS is characterized by a determined unwillingness of a person to engage in activities, including eating, drinking, walking, talking and self-care. The person will withdraw socially and exhibits anger and active resistance against attempts to provide care. 75% of the PRS cases occur in girls aged 8 to 15, with a mean age of 10.5, and the disorder can be life-threatening.<ref name=ecap18_11_645/><ref name=apt10_153/>


A family with a psychiatric history or environmental stress factors can also play a role. Hospitalization is almost always necessary and the recovery period is lengthy; typically 12.8 months. During the recovery period symptoms disappear in the opposite order they appear. That is to say that if food refusal was the first symptom to appear then it would be the last to disappear and if another symptom appears later on, it will disappear relatively early in the recovery process.<ref>{{cite web|last=Lask|first=B.|title=Pervasive refusal syndrome|work=Advances in Psychiatric Treatment|pages=153–159|doi=10.1192/apt.10.2.153|date=NaN undefined NaN}}</ref> About 67% of the cases show complete recovery.<ref name=ecap18_11_645/>
A family with a psychiatric history or environmental stress factors can also play a role. Hospitalization is almost always necessary and the recovery period is lengthy; typically 12.8 months. During the recovery period symptoms disappear in the opposite order they appear. That is to say that if food refusal was the first symptom to appear then it would be the last to disappear and if another symptom appears later on, it will disappear relatively early in the recovery process.<ref>{{cite web|last=Lask|first=B.|title=Pervasive refusal syndrome|work=Advances in Psychiatric Treatment|pages=153–159|doi=10.1192/apt.10.2.153|date=NaN undefined NaN}}</ref> About 67% of the cases show complete recovery.<ref name=ecap18_11_645/>


PRS may be linked to [[learned helplessness]], and so it can be important for the patient to be able to manage the rate of their recovery. [[Music therapy]] may help in this regard as it provides empowerment by giving the patient choice and control, while allowing for [[Musical improvisation|improvisation]] can result in a sense of affirmation and validation; all important for a successful recovery.<ref name=ajmt17_35/>
PRS may be linked to [[learned helplessness]], and so it can be important for the patient to be able to manage the rate of their recovery. [[Music therapy]] may help in this regard as it provides empowerment by giving the patient choice and control, while allowing for [[Musical improvisation|improvisation]] can result in a sense of affirmation and validation; all important for a successful recovery.<ref name=ajmt17_35/>
[[File:Bundesarchiv Bild 183-K0710-0001-011, Mittenwalde, Mittagsruhe im Kindergarten.jpg|thumb|right|Children suffering from PRS often abandon their involvement in all phases of their life.]]




==Epidemiology==
Pervasive refusal syndrome is for the most part frequently seen in girls and less so in boys. The average age of onset is between the ages of 7 and 15.<ref name=Diagnosis /> Affected children are usually high achievers with high self-expectaions, fears of [[Failure|failure]], and difficulty dealing with [[failure]] to achieve personal standards<ref name="Life cycles">{{cite book|last=Judith,|first=S.|title=Essentials of Life Cycle Nutrition|year=2010|publisher=Jones and Bartlett Publishers, LLC.|location=Canada|pages=188|url=http://books.google.ca/books?id=xPyuUPlX7FMC&dq=pervasive+refusal+syndrome+prevalence&source=gbs_navlinks_s|coauthors=Edelstein, S.}}</ref> . The onset of PRS is usually acute<ref name="Life cycles" /> .

==Symptoms==
[[File:Depression-loss of loved one.jpg|thumb|right|Children and adolescent who suffer from PRS often withdraw from social interactions]]
PRS symptoms have common characteristics with many other psychiatric disorders. However, none of the present [[DSM-IV|DSM]] diagnoses can account for the full scope of symptoms seen in PRS, and refusal to eat, weight loss, social withdrawal, school refusal can be considered as the main distinctive features<ref name=Diagnosis /> . Any system may be involved, however some more commonly engaged than others. <ref name=symptoms>{{cite book|last=Coghill|first=David|title=Oxford University Press Amazon.ca Chapters.indigo.ca Find in a library All sellers » New! Shop for Books on Google Play Browse the world's largest eBookstore and start reading today on the web, tablet, phone, or ereader. Go to Google Play Now » My library My History Books on Google Play Child and Adolescent Psychiatry|year=2009|publisher=Oxford University Press.|pages=310|url=http://books.google.ca/books?id=pcUeLW9O0ZgC&dq=pervasive+refusal+syndrome+symptoms&source=gbs_navlinks_s|coauthors=Sally Bonnar, Sandra Duke, Sarah Seth, Johnny Graham}}</ref>

'''[[Gastrointestinal]]''':<ref name=symptoms />
*recurring pain
*[[nausea]]
*loss of appetite
'''[[Neurologic|Neurological]]''':<ref name=symptoms />
*headache
*[[seizure]]
*motor dysfunction
*sensory dysfunction
*[[fatigue]]
*altered [[Consciousness|consciousness]]
'''[[Musculoskeletal]]'''<ref name=symptoms /> :
*joint pains
*muscle weakness

==Etiology==
Trauma, in general, appears to be a vital etiological aspect, due to the fact that PRS is also repeatedly seen in [[Refugee|refugees]] and witnesses to violence<ref name=Diagnosis /> . The helplessness and hopelessness can transmit from parents to children and from children to parents as they watch one another battling with uncontrollable proceedings<ref name=Diagnosis /> . [[Viral infections]] are repeatedly seen to be a factor in PRS, many cases are thought to begin with a viral infection. There have been other theories regarding the etiology of PRS, for instance, the [[Psychodynamics|psychodynamic]] theory of fatal mothering and a potential neurobiological role of the [[Insula|insula]]<ref name=Diagnosis /> . Von Folsach and Montgomery put forth four essential etiologic factors: (1) a premorbid personality, (2) a history of child psychiatric problems, (3) parental psychiatric problems and (4) sudden stressful events<ref name=Diagnosis /> . PRS children are typically known to be [[perfectionists]], conscientious and high achievers. When these children are put in stressful events that they feel they cannot control, they go into into a state of [[learned helplessness]]. Previous child psychiatric problems can designate a susceptibility to develop PRS when put in stressful situations, and parental psychiatric problems may influence the parents’ capability to support and care for their children<ref name=Diagnosis /> .

===Risk Factors for PRS===
Pervasive refusal syndrome is more prone in some people, these risk factors include<ref name="risk factors">{{cite journal|last=Searcy|first=Eileen|title=Helping the patient who has pervasive development disorder|journal=JAAPA : Journal of the American Academy of Physician Assistants|year=2001|volume=14|issue=10|pages=39|url=http://search.proquest.com.myaccess.library.utoronto.ca/docview/232486468}}</ref> :
*[[Fragile X syndrome]]
* Maternal hypothyroidism
*[[Phenylketonuria]]
*[[prenatal]] drug and alcohol exposure
*prenatal viral infection (cytomegalovirus, herpes, measles, syphyilis, toxoplasmosis, etc)
*sibling who has a pervasive developmental disorder
*[[Tuberous sclerosis]]
<ref name="risk factors" />

==Diagnosis==
Thompson and Nunn were the first to introduce diagnostic criteria for PRS in 1997. The current diagnostic criteria consists of:<ref name=Diagnosis>{{cite journal|last=Jaspers|first=Tine|coauthors=G. M. J. Hanssen, Judith A. van der Valk, Johann H. Hanekom, Gijs Th. J. van Well, Jan N. M. Schieveld|title=Pervasive refusal syndrome as part of the refusal–withdrawal– regression spectrum: critical review of the literature illustrated by a case report|journal=Eur Child Adolesc Psychiatry|year=2009|issue=18|pages=645-651|url=http://www-ncbi-nlm-nih-gov.myaccess.library.utoronto.ca/pmc/articles/PMC2762526/pdf/787_2009_Article_27.pdf}}</ref>
* A) Partial or complete refusal in three or more of the following
areas: (1) [[Eating|eating]], (2) [[Mobilization|mobilization]], (3) [[Speech|speech]], (4) interest to
personal care
* B) Active and angry resistance to acts of help and support
* C) [[Social withdrawal]] and school refusal
* D) No organic condition accounts for the severity of the degree of
[[Symptom|symptoms]]
* E) No other [[Psychiatric disorder|psychiatric disorder]] could better account for the
symptoms
* F) The endangered state of the patient requires hospitalization<ref name=Diagnosis />

==Learned Helplessness Model==
[[Learned helplessness|Seligman's]] (1990) model of [[learned helplessness]] embodies that the learned expectation of having no control over matters in the environment is met with a generalized passivity response<ref name=Helplessnes>{{cite book|last=Magagna|first=J.|title=The Silent Child: Communication Without Words|year=2010|publisher=Karnac Books|location=London|pages=141|url=http://books.google.ca/books?id=fflw-NRtuRQC&dq=pervasive+refusal+syndrome+and+learnt+helplessness&source=gbs_navlinks_s}}</ref> . It is speculated that the interactions between the child and events in their surroundings can end in the child encountering feelings of helplessness and therefor, a loss of personal hopefulness<ref name=Helplessnes /> . The patient is scared to take part in the world and feels inadequate in facing [[Internal|internal]] and [[external]] experiences, which he or she experiences through the world and thinking about his or her emotional encounters<ref name=Helplessnes /> . This model is effective in explaining the degradation in children with PRS when trying to rehabilitate them. If the child or adolescent is experiencing the treatment intervention as forceful, then their feeling of helplessness increases<ref name=Helplessnes /> .

==Comorbidity==
{{Main|Autism}}
===Autism and PRS===
[[File:Autism-stacking-cans.jpg|thumb|right|Children with autism often engage in restircted and repetitive behavior]]
[[Autism]] is a [[Autism|neural development disorder]] defined by flawed [[Social interaction|social interaction]] and [[Communication|communication]], and by restricted and repetitive behavior<ref name="risk factors" /> . The patient with autism displays substantial deficit in all three core deficits of PRS which include: impairments in social interaction, impairments in communication, and restricted and stereotyped patterns of behavior, interests, and activities<ref name="risk factors" /> . The patient who has autistic disorder is usually mentally [[Retarded|retarded]] as well<ref name="risk factors" /> .

{{Main|Asperger syndrome}}
===Asperger's syndrome and PRS===
[[Asperger's syndrome]] (AS) is characterized by considerable problems in social interaction, other notable symptoms include restricted and repetitive patterns of behavior and activities<ref name="risk factors" /> . Patient with AS generally has no setback in language [[Cognitive neuroscience|cognitive]] maturity, or self-help abilities but has clear [[Language deficits|language skill deficits]], problems in social interaction, and odd behavior in interests and activities characteristic of PRS<ref name="risk factors" /> . The lack of cognitive development deficits enables the patient with AS to perform at a more advanced level than people who have other forms of PRS<ref name="risk factors" /> .

==Treatment==
Unfortunately, no evidence-based treatment is known for PRS. However it is widely accepted that the treatment must incorporate a complete [[Multidisciplinary approach|multidisciplinary]] team approach and a controlled yet flexible management plan with a visible basis engaged over months to years<ref name=Diagnosis /> . Recovery from pervasive refusal syndrome is slow, usually demands one year after diagnosis and introduction of treatment, but many children have a complete recovery and relapse is almost never seen<ref name="Life cycles" /> .

===Inpatient Treatment===
Due to the fact that PRS is such a severe disorder, it is almost always required to [[Hospitalization|hospitalize]] in a child and adolescent [[Psychiatric Unit|psychiatric unit]]<ref name=Diagnosis /> . [[Outpatient care|Outpatient]] treatment does display symptom-free periods, but [[Relapse|relapses]] of short-lived episodes of depressive symptoms or [[anorexia]] are observed<ref name=Diagnosis /> . It is therefore necessary to partake in inpatient treatment. Treatment ought to involve gentle loving care. The person treating the patient must be very [[Sensitivity|sensitive]] and tolerant because it takes a long period of time for the patient to get better, and putting pressure on them adds severity to their condition<ref name=Diagnosis /> . It frequently takes several months of treatment before it is likely to employ a very steady [[Rehabilitation (neuropsychology)|rehabilitation programme]]<ref name=Diagnosis /> .

===Role of the Family===

The role of the family in the [[Treatment of mental disorders|treatment]] process is vital yet complicated, given that withdrawal of the child from [[Therapy|therapy]] is a key problem. It is important to include the family of the patient in the treatment process as it eases family [[anxiety]] and distress<ref name=Diagnosis /> . Nonetheless, it is important to create some space because too much involvement of the family may be counterproductive. [[Medication]] seems to play a very restricted part in the management of pervasive refusal syndrome (PRS), having importance in the treatment of comorbid disorders only, for example [[Antidepressant|antidepressants]] for [[Comorbidity|comorbid]] depression<ref name=Diagnosis /> .


==References==
==References==

Revision as of 19:06, 17 August 2012

Pervasive refusal syndrome (PRS) is a rare but serious child psychiatric disorder that was first described by Bryan Lask and colleagues in 1991.[1] As of 2011, it is not included in the standard psychiatric classification systems.[2]. PRS is the name allotted to a disorder in which children have abandoned their involvement in all phases of their life. It's characterized by refusal to eat, drink, talk, walk or self-care, and a firm resistance to treatment [3]. PRS is very rare and its cause is unclear, but its severity makes it life-threatening. The disorder usually begins with a 'virus', or the child having a 'pain', that results in the need for consulting a doctor or going to the hospital, even though no substantial cause can be found. PRS starts slowly, but the child then worsens quickly becoming reluctant or not capable to do anything for themselves [3] . They originally refuse to accept others caring for them, or helping them eat, and are very depressed and distraught. It is not guaranteed that recovery will take place, and it is a lengthy and complex process, involving specialist medical care [3] . Nevertheless, once the patient is healthy, relapse is very infrequent.

A family with a psychiatric history or environmental stress factors can also play a role. Hospitalization is almost always necessary and the recovery period is lengthy; typically 12.8 months. During the recovery period symptoms disappear in the opposite order they appear. That is to say that if food refusal was the first symptom to appear then it would be the last to disappear and if another symptom appears later on, it will disappear relatively early in the recovery process.[4] About 67% of the cases show complete recovery.[5]

PRS may be linked to learned helplessness, and so it can be important for the patient to be able to manage the rate of their recovery. Music therapy may help in this regard as it provides empowerment by giving the patient choice and control, while allowing for improvisation can result in a sense of affirmation and validation; all important for a successful recovery.[6]

Children suffering from PRS often abandon their involvement in all phases of their life.



Epidemiology

Pervasive refusal syndrome is for the most part frequently seen in girls and less so in boys. The average age of onset is between the ages of 7 and 15.[7] Affected children are usually high achievers with high self-expectaions, fears of failure, and difficulty dealing with failure to achieve personal standards[8] . The onset of PRS is usually acute[8] .

Symptoms

Children and adolescent who suffer from PRS often withdraw from social interactions

PRS symptoms have common characteristics with many other psychiatric disorders. However, none of the present DSM diagnoses can account for the full scope of symptoms seen in PRS, and refusal to eat, weight loss, social withdrawal, school refusal can be considered as the main distinctive features[7] . Any system may be involved, however some more commonly engaged than others. [9]

Gastrointestinal:[9]

  • recurring pain
  • nausea
  • loss of appetite

Neurological:[9]

Musculoskeletal[9] :

  • joint pains
  • muscle weakness

Etiology

Trauma, in general, appears to be a vital etiological aspect, due to the fact that PRS is also repeatedly seen in refugees and witnesses to violence[7] . The helplessness and hopelessness can transmit from parents to children and from children to parents as they watch one another battling with uncontrollable proceedings[7] . Viral infections are repeatedly seen to be a factor in PRS, many cases are thought to begin with a viral infection. There have been other theories regarding the etiology of PRS, for instance, the psychodynamic theory of fatal mothering and a potential neurobiological role of the insula[7] . Von Folsach and Montgomery put forth four essential etiologic factors: (1) a premorbid personality, (2) a history of child psychiatric problems, (3) parental psychiatric problems and (4) sudden stressful events[7] . PRS children are typically known to be perfectionists, conscientious and high achievers. When these children are put in stressful events that they feel they cannot control, they go into into a state of learned helplessness. Previous child psychiatric problems can designate a susceptibility to develop PRS when put in stressful situations, and parental psychiatric problems may influence the parents’ capability to support and care for their children[7] .

Risk Factors for PRS

Pervasive refusal syndrome is more prone in some people, these risk factors include[10] :

[10]

Diagnosis

Thompson and Nunn were the first to introduce diagnostic criteria for PRS in 1997. The current diagnostic criteria consists of:[7]

  • A) Partial or complete refusal in three or more of the following

areas: (1) eating, (2) mobilization, (3) speech, (4) interest to personal care

  • B) Active and angry resistance to acts of help and support
  • C) Social withdrawal and school refusal
  • D) No organic condition accounts for the severity of the degree of

symptoms

symptoms

  • F) The endangered state of the patient requires hospitalization[7]

Learned Helplessness Model

Seligman's (1990) model of learned helplessness embodies that the learned expectation of having no control over matters in the environment is met with a generalized passivity response[11] . It is speculated that the interactions between the child and events in their surroundings can end in the child encountering feelings of helplessness and therefor, a loss of personal hopefulness[11] . The patient is scared to take part in the world and feels inadequate in facing internal and external experiences, which he or she experiences through the world and thinking about his or her emotional encounters[11] . This model is effective in explaining the degradation in children with PRS when trying to rehabilitate them. If the child or adolescent is experiencing the treatment intervention as forceful, then their feeling of helplessness increases[11] .

Comorbidity

Autism and PRS

Children with autism often engage in restircted and repetitive behavior

Autism is a neural development disorder defined by flawed social interaction and communication, and by restricted and repetitive behavior[10] . The patient with autism displays substantial deficit in all three core deficits of PRS which include: impairments in social interaction, impairments in communication, and restricted and stereotyped patterns of behavior, interests, and activities[10] . The patient who has autistic disorder is usually mentally retarded as well[10] .

Asperger's syndrome and PRS

Asperger's syndrome (AS) is characterized by considerable problems in social interaction, other notable symptoms include restricted and repetitive patterns of behavior and activities[10] . Patient with AS generally has no setback in language cognitive maturity, or self-help abilities but has clear language skill deficits, problems in social interaction, and odd behavior in interests and activities characteristic of PRS[10] . The lack of cognitive development deficits enables the patient with AS to perform at a more advanced level than people who have other forms of PRS[10] .

Treatment

Unfortunately, no evidence-based treatment is known for PRS. However it is widely accepted that the treatment must incorporate a complete multidisciplinary team approach and a controlled yet flexible management plan with a visible basis engaged over months to years[7] . Recovery from pervasive refusal syndrome is slow, usually demands one year after diagnosis and introduction of treatment, but many children have a complete recovery and relapse is almost never seen[8] .

Inpatient Treatment

Due to the fact that PRS is such a severe disorder, it is almost always required to hospitalize in a child and adolescent psychiatric unit[7] . Outpatient treatment does display symptom-free periods, but relapses of short-lived episodes of depressive symptoms or anorexia are observed[7] . It is therefore necessary to partake in inpatient treatment. Treatment ought to involve gentle loving care. The person treating the patient must be very sensitive and tolerant because it takes a long period of time for the patient to get better, and putting pressure on them adds severity to their condition[7] . It frequently takes several months of treatment before it is likely to employ a very steady rehabilitation programme[7] .

Role of the Family

The role of the family in the treatment process is vital yet complicated, given that withdrawal of the child from therapy is a key problem. It is important to include the family of the patient in the treatment process as it eases family anxiety and distress[7] . Nonetheless, it is important to create some space because too much involvement of the family may be counterproductive. Medication seems to play a very restricted part in the management of pervasive refusal syndrome (PRS), having importance in the treatment of comorbid disorders only, for example antidepressants for comorbid depression[7] .

References

  1. ^ Lask B, Britten C, Kroll L, Magagna J, Tranter M (1991). "Children with pervasive refusal". Archives of Disease in Childhood. 66: 866–869. doi:10.1136/adc.66.7.866.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  2. ^ Grahame V, Reid C, Rao S, Guirguis S, Kaplan, C (2011). "P01-290 - Pervasive refusal syndrome: comparing and contrasting clinical cases". European Psychiatry. 26: 291. doi:10.1016/S0924-9338(11)72001-6. {{cite journal}}: Unknown parameter |supplement= ignored (help)CS1 maint: multiple names: authors list (link)
  3. ^ a b c "Pervasive Refusal Syndrome: A Parent's Perspective". Sage Journals. 6 (455). 2001.
  4. ^ Lask, B. (NaN undefined NaN). "Pervasive refusal syndrome". Advances in Psychiatric Treatment. pp. 153–159. doi:10.1192/apt.10.2.153. {{cite web}}: Check date values in: |date= (help); Missing or empty |url= (help)
  5. ^ Jaspers T, Hanssen GM, van der Valk JA, Hanekom JH, van Well GT, Schieveld JN (2009). "Pervasive refusal syndrome as part of the refusal-withdrawal-regression spectrum: critical review of the literature illustrated by a case report". European Child & Adolescent Psychiatry. 18 (11): 645–651. doi:10.1007/s00787-009-0027-6. {{cite journal}}: Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  6. ^ van der Walt M, Annette B (2006). "The Role of Music Therapy in the Treatment of a Girl with Pervasive Refusal Syndrome: Exploring Approaches to Empowerment". Australian Journal of Music Therapy. 17: 35–53. Retrieved 2011-06-11.
  7. ^ a b c d e f g h i j k l m n o p Jaspers, Tine (2009). "Pervasive refusal syndrome as part of the refusal–withdrawal– regression spectrum: critical review of the literature illustrated by a case report" (PDF). Eur Child Adolesc Psychiatry (18): 645–651. {{cite journal}}: Unknown parameter |coauthors= ignored (|author= suggested) (help)
  8. ^ a b c Judith,, S. (2010). Essentials of Life Cycle Nutrition. Canada: Jones and Bartlett Publishers, LLC. p. 188. {{cite book}}: Unknown parameter |coauthors= ignored (|author= suggested) (help)CS1 maint: extra punctuation (link)
  9. ^ a b c d Coghill, David (2009). Oxford University Press Amazon.ca Chapters.indigo.ca Find in a library All sellers » New! Shop for Books on Google Play Browse the world's largest eBookstore and start reading today on the web, tablet, phone, or ereader. Go to Google Play Now » My library My History Books on Google Play Child and Adolescent Psychiatry. Oxford University Press. p. 310. {{cite book}}: Unknown parameter |coauthors= ignored (|author= suggested) (help); horizontal tab character in |title= at position 307 (help)
  10. ^ a b c d e f g h Searcy, Eileen (2001). "Helping the patient who has pervasive development disorder". JAAPA : Journal of the American Academy of Physician Assistants. 14 (10): 39.
  11. ^ a b c d Magagna, J. (2010). The Silent Child: Communication Without Words. London: Karnac Books. p. 141.
Cite error: A list-defined reference named "apt10_153" is not used in the content (see the help page).